Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2

被引：4

作者：

Czubkowski, Piotr ^{[1
]}

Jankowska, Irena ^{[1
]}

Pawlowska, Joanna ^{[1
]}

机构：

[1] Childrens Mem Hlth Inst, Dept Gastroenterol Hepatol Feeding Disorders & Pe, PL-04730 Warsaw, Poland

来源：

ANNALS OF HEPATOLOGY | 2015年 / 14卷 / 04期

关键词：

Partial external biliary diversion; Quality of life; Maternity; External stoma; Bile acids; SALT EXPORT PUMP; PRURITUS; DISEASE; BILE; 4-PHENYLBUTYRATE; DIVERSION; CHILDREN; THERAPY; BSEP;

D O I：

10.1016/S1665-2681(19)31178-0

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Progressive familial intrahepatic cholestasis type 2 (PFIC 2) results from mutations in ABCB11 gene coding bile salt export pump (BSEP). Medical treatment is usually unsuccessful and surgery intervention is necessary. Partial external biliary diversion (PEBD) is regarded as the first choice of surgical treatment. Real exclusion (IE) is an alternative operation if external stoma is not tolerated; however, a favorable outcome is uncertain. In chronic liver diseases pregnancy brings additional risk of deterioration of liver function and generally is not recommended. We present the first case report of successful pregnancy in a genetically confirmed PFIC 2 patient after surgical conversion from PEBD to IE.

引用

页码：550 / 552

页数：3

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